3B14.0

ICD-11 MMS 2026-01

Hereditary deficiency of factor I

Description

Congenital deficiencies of fibrinogen are coagulation disorders characterised by bleeding symptoms ranging from mild to severe resulting from reduced quantity and/or quality of circulating fibrinogen. Afibrinogenaemia (complete absence of fibrinogen) and hypofibrinogenaemia (reduced plasma fibrinogen concentration) correspond to quantitative anomalies of fibrinogen while dysfibrinogenaemia corresponds to a functional anomaly of fibrinogen. Hypo- and dysfibrinogenaemia may be frequently combined (hypodysfibrinogenaemia).

Index terms

  • Hereditary deficiency of factor I
  • Deficiency of factor 1
  • Deficiency of fibrinogen
Show allHide
  • Hereditary fibrinogen deficiency
  • congenital fibrinogenopenia
  • Hereditary afibrinogenaemia
  • Congenital afibrinogenaemia
  • Hereditary dysfibrinogenaemia
  • congenital dysfibrinogenaemia
  • dysfibrinogenaemia NOS
  • Hereditary hypofibrinogenaemia
  • congenital hypofibrinogenaemia
  • Hereditary hypodysfibrinogenaemia

Exclusions from above levels

  • JA43.3 – Postpartum coagulation defects
  • 04 – Diseases of the immune system
  • 05 – Endocrine, nutritional or metabolic diseases
Show allHide
  • 18 – Complications of pregnancy, childbirth or the puerperium
  • 19 – Certain conditions originating in the perinatal period
  • 20 – Congenital malformations, deformations or chromosomal abnormalities
  • 22 – Injury, poisoning or certain other consequences of external causes
  • http://id.who.int/icd/entity/1000704511 – Human immunodeficiency virus disease
  • JB64.1 – Other diseases of the blood or blood-forming organs or certain disorders involving the immune mechanism complicating pregnancy, childbirth or the puerperium

Sibling codes

  • 3B14.1 – Hereditary factor X deficiency
  • 3B14.2 – Combined deficiency of vitamin K-dependent clotting factors
  • 3B14.Z – Other inherited coagulation factor deficiency with bleeding tendency, unspecified

Foundation entity

Hereditary deficiency of factor I – http://id.who.int/icd/entity/1452989457

Part of: 3B14: Other inherited coagulation factor deficiency with bleeding tendency › 03: Diseases of the blood or blood-forming organs